Volume 6 Issue 12
Dec.  2015
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Chao Chen, Ye Chen, Min-Xin Guan. A peep into mitochondrial disorder: multifaceted from mitochondrial DNA mutations to nuclear gene modulation[J]. Protein&Cell, 2015, 6(12): 862-870. doi: 10.1007/s13238-015-0175-z
Citation: Chao Chen, Ye Chen, Min-Xin Guan. A peep into mitochondrial disorder: multifaceted from mitochondrial DNA mutations to nuclear gene modulation[J]. Protein&Cell, 2015, 6(12): 862-870. doi: 10.1007/s13238-015-0175-z

A peep into mitochondrial disorder: multifaceted from mitochondrial DNA mutations to nuclear gene modulation

doi: 10.1007/s13238-015-0175-z
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The authors were supported by the National Basic Research Program (973 Program) (No. 2014CB541700), the project from National Natural Science Foundation of China (Grant No. 81470685) and the Natural Science Foundation of Zhejiang province (LR15H080001).

  • Received Date: 2015-03-18
  • Rev Recd Date: 2015-05-17
  • Mitochondrial genome is responsible for multiple human diseases in a maternal inherited pattern, yet phenotypes of patients in a same pedigree frequently vary largely. Genes involving in epigenetic modification, RNA processing, and other biological pathways, rather than "threshold effect" and environmental factors, provide more specific explanation to the aberrant phenotype. Thus, the double hit theory, mutations both in mitochondrial DNA and modifying genes aggravating the symptom, throws new light on mitochondrial dysfunction processes. In addition, mitochondrial retrograde signaling pathway that leads to reconfiguration of cell metabolism to adapt defects in mitochondria may as well play an active role. Here we review selected examples of modifier genes and mitochondrial retrograde signaling in mitochondrial disorders, which refine our understanding and will guide the rational design of clinical therapies.
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